Table I.
Maternal outcomes of the 39 pregnancies.
| No. | Variant(s) | CKD prior to P | Complicated/total P | Adverse event | Treatment of P‐aHUS | Sequelae | Follow‐up,* years | Age, years | CKD at last visit |
|---|---|---|---|---|---|---|---|---|---|
| Primary aHUS prior to first pregnancy | |||||||||
| B5 | C3 c.3125G>A2 | – | 0/2 | – | N/a | – | 4 | 28 | – |
| P‐Ahus | |||||||||
| M1 | C3 c.481C>T1 | – | 3/3 | HT, P‐aHUS (+60 days) | PEX | ESKD | 18 | 47 | G5+/T |
| M5 | – | – | 6/7 | PE, P‐aHUS (+2 days) | – | CKD G4 | 16 | 35 | ESKD |
| G4 | 7/7 | PE, bleeding | N/a | ESKD | |||||
| M9 | – | – | 1/1 | P‐aHUS (+0 day), bleeding | PEX, Ecu | – | 3 | 30 | – |
| M11 | – | – | 1/1 | PE, P‐aHUS (+0 day) | PEX, Ecu | – | 1 | 30 | – |
| B1 | – | – | 1/2 | PE | N/a | HT | 13 | 39 | – |
| 2/2 | HELLP, P‐aHUS (+0 day) | PEX | – | ||||||
| B2 | – | – | 1/1 | PE, P‐aHUS (+0 day) | PEX | – | 1 | 30 | – |
| B6 | CFI c.772G>A2 | – | 1/1 | PE, P‐aHUS (+1 day) | PEX, Ecu | ESKD | 1 | 32 | ESKD |
| Primary aHUS after last pregnancy | |||||||||
| M2 | CFI c.1420C>T1 | ||||||||
| C3 c.463A>C1 | – | 0/1 | – | N/a | – | 36 | 62 | G5+/T | |
| M3 | C3 c.481C>T1 | – | 1/1 | PE | N/a | Unknown | 14 | 49 | G3/T |
| M4 | CFH c.2558G>A1 | – | 0/1 | – | N/a | – | 10 | 40 | G3/T |
| M6 | C3 c.481C>T1 | – | 1/1 | HELLP | N/a | Unknown | 7 | 32 | G2/T |
| M7 | – | – | 1/4 | PE | N/a | None | 45 | 74 | G4 |
| M8 | – | – | 0/4 | – | N/a | – | 21 | 46 | ESKD |
| M10 | – | – | 3/3 | HT | N/a | HT | 49 | 74 | G3 |
| B3 | CFH c.3486delA1 | – | 0/1 | – | N/a | – | 2 | 32 | – |
| B4 | – | – | 0/2 | – | N/a | – | 46 | 73 | G3 |
| Asymptomatic relative | |||||||||
| M12 | CD46 c.811_816delGACAGT1 | – | 0/1 | – | N/a | – | 50 | 82 | G3 |
| B7 | CD46 c.811_816delGACAGT1 | – | 0/2 | – | N/a | – | 6 | 37 | – |
aHUS, atypical haemolytic uraemic syndrome; B1–7, Brussels cohort; CKD, chronic kidney disease (T, transplantation); Ecu, eculizumab; ESKD, end‐stage kidney disease; HELLP, haemolysis, elevated liver enzymes, low platelets; HT, (gestational) hypertension; M1–12, Maastricht cohort; P, pregnancy; PE, pre‐eclampsia; PEX, plasma exchange.
*
Follow‐up after the first pregnancy. Rare variants in complement genes were classified as 1pathogenic or 2uncertain significance.